Coexistent Ampullary Squamous Cell Carcinoma with Adenocarcinoma of the Pancreatic Duct

Primary squamous cell carcinoma (SCC) of ampulla has seldom been reported. However, metastatic SCC to ampulla of Vater is well known. We report a case of primary SCC of ampulla of Vater coexistent with well-differentiated adenocarcinoma of the distal pancreatic duct. A 50-year-old female presented with evidence of obstructive jaundice. Endoscopic retrograde cholangio-pancreatography revealed bulging papilla with ulcero-infiltrative growth at the ampulla of Vater. An initial endoscopic biopsy of the ampullary mass showed a well-differentiated SCC. The patient underwent Whipple''s operation. Thorough sampling of the dilated portion of the pancreatic duct showed presence of well-differentiated adenocarcinoma of the distal pancreatic duct. Immunohistochemical study with synaptophysin and chromogranin was done with negative result, ruling out neuroendocrine differentiation. Also, a detailed clinical, endoscopic and radiological examination was carried out, that excluded the presence of primary SCC elsewhere.     

Small cell neuroendocrine carcinoma of the ampulla of Vater with foci of squamous differentiation: a case report

A 54-year-old woman with obstructive jaundice was found to have a 4-cm ulcerated, elevated tumor, located at the papilla of Vater by endoscopy and radiographic investigation. Based on a clinical diagnosis of carcinoma of the ampulla of Vater, a pylorus-preserving pancreaticoduodenectomy was performed. The histologic appearance of the lesion was identical to extrapulmonary small cell carcinoma, with diffuse proliferation of small, spindle-shaped, atypical tumor cells with numerous mitoses. Neuroendocrine differentiation was demonstrated by immunoreactivity with neuron-specific enolase (NSE) and Leu-7, and by the presence of dense core granules ultrastructurally. The tumor was composed mainly of small cell neuroendocrine carcinoma, and partially of an area of squamous differentiation, showing transition from one to the other. Histopathologic investigation disclosed direct invasion deep to the lamina propria, and infiltration of the pancreatic parenchyma, duodenum, and bile duct. Lymph node metastases were present in the superior pancreaticoduodenal chain. The patient died of liver metastases 8 months later. As in previously reported cases of small cell neuroendocrine carcinoma of the ampullary region, the present case showed extremely aggressive clinical behavior with early metastases resulting in fatal outcome.     

Signet-ring-cell carcinoma of the ampulla of Vater: a case report

We encountered a rare variant of carcinoma of the ampulla of Vater in a 68-year-old man with postprandial abdominal pain and nausea. Ultrasonography and computed tomography showed a dilated common bile duct and main pancreatic duct. At duodenoscopy, the papilla of Vater was enlarged, and biopsy specimens taken from the papilla revealed signet-ring-cell carcinoma. Endoscopic ultrasonography showed a hypoechoic tumor without pancreatic invasion. Pylorus-preserving pancreatoduodenectomy was performed. Histologic examination of resected specimens indicated lymphatic and vascular invasion, but surgical margins were tumor-free. The patient is alive and disease-free 10 months after the operation.     

Signet-ring cell carcinoma of ampulla of Vater： Contrast-enhanced ultrasound findings

Signet-ring cell carcinoma （SRCC） of ampulla of Vater is extremely uncommon, and less than 15 cases have been reported so far in literature. It mainly occurs in elderly people （median age 57 years）. We report a rare case of SRCC of the ampulla of Vater in a 38-year-old woman who presented with a small tumor at the Vater, discovered by the contrast-enhanced ultrasound （CEUS）. Histopathological examination showed prominent signet-ring features. We also describe the imaging features of SRCC of ampulla of Vater in CEUS.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

Peritoneal recurrence of ampullary carcinoma following curative pancreatoduodenectomy

A 72 year-old Japanese man with peritoneal recurrence of carcinoma of the ampulla of Vater after curative pancreatoduodenectomy is presented. He was treated by percutaneous transhepatic biliary drainage (PTBD) for obstructive jaundice. The PTBD catheter dislodged 14 days later. He underwent emergency open peritoneal lavage and external choledochal drainage for diffuse bile peritonitis. Cytologic examination of bile obtained from the T-tube revealed malignant cells. He underwent pancreatoduodenectomy with regional lymph node dissection 2 months later for ampullary carcinoma. Pathologic examination showed a macroscopic protruding, 8 x 7 x 10 mm, papillary adenocarcinoma of the ampulla of Vater. The tumor was classified as stage II with pT2, pN0, and pM0. Eight months later, cytologic examination of ascites demonstrated adenocarcinoma cells. The patient died with peritoneal recurrence 10 months after curative pancreatoduodenectomy.     

A case of ampullary cancer with pancreas divisum treated by endoscopic papillectomy

A 60's man underwent a medical check-up and esophagogastroduodenoscopy revealed an exposed-type tumor at the ampulla of Vater. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a slightly dilated ventral pancreatic duct not connected to the dorsal duct. Endoscopic papillectomy was performed without pancreatic stent placement and his postprocedural course was uneventful. The specimen was histologically diagnosed as well-differentiated adenocarcinoma limited to the mucosa of the ampulla of Vater. Endoscopic papillectomy without pancreatic stent placement can be performed without a risk of post-ERCP pancreatitis for ampullary tumor limited to the mucosa of the ampulla of Vater associated with pancreas divisum.     

Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.     

Surgical technique for complete resection of the extrahepatic portion of the common bile duct and the ampulla of Vater for tumors of the ampulla of Vater

Background

Ampullary tumors have to be completely resected, but substantial morbidity and mortality rates are associated with pancreaticoduodenectomy (PD). Local resection can be the procedure of choice in selected ampullary lesions for high-risk patients.

Methods

Preoperative examination indicated that the ampullary tumor extended into the common bile duct without evidence of pancreatic duct involvement and no definite invasion into either the duodenum or the pancreas. We performed a complete resection of the extrahepatic bile duct and the ampulla of Vater, including the tumor, without performing PD by dissecting the intrapancreatic bile duct from the pancreas both downward towards the ampulla of Vater and upward using a transduodenal approach.

Results

The operation was successfully completed, and the postoperative course was uneventful, with the exception of a minor pancreatic fistula from retropancreatic dissection. The final pathological examination demonstrated well-differentiated tubular adenocarcinoma limited to the mucosa with negative surgical margins.

Conclusion

Complete resection of the extrahepatic bile duct and the ampulla of Vater through a transduodenal approach can be a feasible and safe surgical procedure for selected ampullary tumors in high-risk patients.     

A case of hepatectomy for liver metastasis after pancreatoduodenectomy for carcinoma of ampulla of Vater]

After curative resection of carcinoma of ampulla of Vater, 5-year survival rate has been reported ranging from 40% to 60%. Two major causes of the treatment failure are local recurrence and liver metastasis. Liver metastases are often multiple and are associated with poor prognosis. There have been few reports on long-term survivors after hepatectomy for metastatic liver tumors from carcinoma of ampulla of Vater. We report a 42 year-old female patient with solitary hepatic metastasis from carcinoma of ampulla of Vater, which was successfully treated by hepatectomy 69 months after curative Whipple's operation. Histologic examination of the resected specimen had revealed stage IB moderately-differentiated, intestinal type adenocarcinoma (T2N0M0). Since neither local recurrence or distant metastasis were detected, the patient underwent liver segmentectomy. Histologic study confirmed the presence of metastatic liver tumor from carcinoma of ampulla of Vater. She is doing well without evidence of recurrence at 20 months after hepatectomy.     

Signet ring cell carcinoma of the ampulla of Vater

Introduction

Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies.

Case report

We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy.

Discussion

Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.     

Undifferentiated carcinoma of the duodenal ampulla

This report demonstrates a case of undifferentiated carcinoma of the duodenal ampulla. A 74-year male experienced jaundice lasting for 3 weeks. An upper gastrointestinal series demonstrated a polypoid, ovoid filling defect in the second portion of the duodenum, and duodenoscopy disclosed a protruding mass involving the orifice of the papilla of Vater. Cholangiography demonstrated obstruction due to compression in the terminal common bile duct. Pylorus-preserving pancreatoduodenectomy was performed on the diagnosis of ampullary carcinoma. The gross specimen showed a polypoid mass, measuring 3.5 cm in diameter, in the ampulla, located mainly in the duodenal submucosal layer and invading the terminal common bile duct. Histologically, the tumor was small cell type, undifferentiated carcinoma, arising from the duodenal epithelium adjacent to the ampulla.     

A rare case of periampullary carcinoma with ectopic ending of Vater＇s ampulla

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 too. Magnetic resonance imaging and magnetic resonance cholangiopancreatography （MRCP） showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm × 5 cm×5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater＇s ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.     

Proposed indications for limited resection of early ampulla of Vater carcinoma: clinico-histopathological criteria to confirm cure

Background

Limited resection is reserved for patients with high operative risk or benign adenomas. We aimed to define indications for limited resection of early ampulla of Vater carcinoma with curative intent through detailed preoperative examinations and histopathological evaluations.

Methods

We performed a retrospective cohort study of all consecutive Japanese patients who underwent resection for ampulla of Vater neoplasms at our hospital from 1986 to 2010.

Results

A total of 75 patients were identified. Moderately/poorly differentiated histology, lympho-vascular/perineural invasion, and duodenal/pancreatic invasion were significant risk factors for lymph node metastases. Macroscopically, non-exposed protruded- or ulcerative-type disease did not correlate directly with lymph node metastases; however, these tumor types were associated with other invasive features. In a subset of early carcinomas fulfilling the conditions of exposed protruded adenoma or papillary/well-differentiated adenocarcinoma determined by endoscopic biopsy, negative duodenal invasion determined by endoscopic ultrasonography, no tumor infiltration into the pancreatic duct determined by intraductal ultrasound, and diameter of the pancreatic duct ≤3?mm determined by endoscopic retrograde cholangiopancreatography (N?=?11), the incidence of lymph node metastasis and tumor infiltration into the pancreatic duct was 0%.

Conclusion

Strictly selected patients with early ampulla of Vater carcinomas may benefit from limited resection if the resected specimen is evaluated to confirm all histopathological criteria.     

Ampullary somatostatinoma in a patient with von Recklinghausen's disease

We report a case of somatostatinoma of the ampulla of Vater associated with von Recklinghausen's disease in a 44-year-old woman. On admission the patient was jaundiced, and percutaneous Cholangiodrainage was performed. Cholangiography revealed stenosis of the common bile duct at the lower end Duodenoscopy showed a yellowish tumor of the ampulla of Vater, and the biopsy specimens showed no malignant cells. Pylorus-preserving pancreaticoduodenectomy was performed. Histologically, the tumor was composed of small round cells with a solid or trabecular pattern and with multiple psammoma bodies. Immunohistochemical examination showed that the tumor cells stained for somatostatin. Genomic examination showed neither K-ras nor p53 gene mutations of the resected specimen.     

KL-6 mucin expression in carcinoma of the ampulla of Vater： Association with cancer progression

AIM: To assess histochemical expression of KL-6 and its clinicopathological significance in carcinoma of the ampulla of Vater. METHODS: Ampullary carcinoma tissues were collected from 38 patients who underwent pancreatoduodenectomy or local resection. Tissues were subjected to immunohistochemical analysis using KL-6 antibody. RESULTS: Positive staining of ampullary carcinoma cells was observed in 26 (68.4%) cases. Staining was not found in the surrounding non-cancer regions of the ampullary tissues. Remarkable KL-6 expression was observed in invasive carcinoma cells in pancreatic and duodenal tissues and in metastatic carcinoma cells in lymph nodes. Positive KL-6 expression was related to lymph node metastasis (P=0.020), pancreatic invasion (P=0.016), duodenal invasion (P= 0.034), and advanced stage of TNM clinical classification (P=0.010). Survival analysis showed that positive expression of KL-6 was related to a poorer prognosis (P=0.029). CONCLUSION: The aberrant expression of KL-6 mucin is significantly related to unfavorable behaviors of carcinoma of the ampulla of Vater.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

Resection of a non-functioning islet cell carcinoma occupying the entire pancreas

A 46-year-old woman with jaundice was found to have enlargement of the entire pancreas on computed tomography. Endoscopy revealed a tumor at the papilla of Vater, although examination of biopsy specimens did not demonstrate a malignancy. The jaundice resolved spontaneously, but reappeared at 7 months. Also noted was increased swelling of the papilla. The serum pancreatic endocrine and exocrine hormone concentrations were within the reference ranges. At surgery, a hard mass was found occupying the entire pancreas. Examination of incisional biopsy specimens from the pancreatic mass and papilla of Vater revealed an islet cell tumor. A total pancreatectomy was performed. The tumor extended from the pancreatic tail to the papilla of Vater. Histopathologic and immunopathologic examination revealed a non-functional islet cell carcinoma. There was no evidence of recurrence at 3 years. A good outcome can be achieved with resection of non-functioning islet cell carcinomas, even if they are advanced.     

Hemobilia due to biliary intraepithelial neoplasia associated with Zollinger–Ellison syndrome

A 58-year-old man was transferred to us from his local hospital because of failure to control his gastrointestinal bleeding by endoscopic hemostasis. Abdominal imaging suggested a hypervascular tumor of the pancreatic head (36?mm diameter), and laboratory testing showed an elevated serum gastrin level (17,800?pg/mL). Gastroduodenal endoscopy revealed multiple duodenal ulcers and active bleeding from the ampulla of Vater. The selective arterial secretagogue injection test suggested a gastrinoma in the pancreatic head, but no gastrinoma in the pancreatic tail. The patient was diagnosed with solitary pancreatic head gastrinoma complicated by hemosuccus pancreaticus, and pancreaticoduodenectomy was performed. Intraoperatively, the diagnosis was changed to primary peripancreatic lymph node gastrinoma without pancreatic involvement. The gastrointestinal bleeding stopped postoperatively and serum gastrin levels returned to normal. Histological examination of the surgical specimens revealed a small submucosal gastrinoma in the duodenum (7?mm diameter). The final diagnosis was microgastrinoma of the duodenum with peripancreatic lymph node metastasis. The cause of bleeding from the ampulla of Vater was initially obscure, but eventually a hemorrhagic erosion with moderate atypia was found in the common bile duct, indicating biliary intraepithelial neoplasia (BilIN). This is the first report of hemobilia due to BilIN with gastrinoma.     

Adenosquamous carcinoma of the pancreas

A 58-year-old Japanese man was admitted complaining of abdominal pain. An abdominal computed tomography examination demonstrated a tumor in the head of the pancreas and multiple calcifications. A laparotomy was performed and the tumor was removed by Whipple's operation. Histologically, the neoplasm that invaded the duodenal wall and the papilla of Vater was composed of nests of malignant squamous cells with intercellular bridges and showed the formation of keratinized pearls with a small area of concurrently neoplastic glandular and squamous elements. On the basis of these features, the diagnosis of adenosquamous carcinoma of the pancreas was made. The patient died 18 months after the operation. The neoplastic behavior of this rare primary pancreatic carcinoma is similar to that of duct cell carcinoma as well as pure squamous cell carcinoma of the pancreas. As the pancreas can be the target of metastases of squamous carcinomas from other organs it is wise to be aware of this rare entity.